Evidence-Based Guidelines for Acute Stabilization and Management of Neonates with Persistent Pulmonary Hypertension of the Newborn

Ball, Molly K.; Seabrook, Ruth; Bonachea, Elizabeth; Chen, Bernadette; Fathi, Omid; Nankervis, Craig A.; Osman, Ahmed; Schlegel, Amy; Magers, Jacqueline; Kulpa, Taylor; Sharpin, Paula; Snyder, Mary Lindsay; Gajarski, Robert J.; Nandi, Deipanjan; Backes, Carl H.

doi:10.1055/a-1711-0778

Cited by 8 publications

(4 citation statements)

References 116 publications

(238 reference statements)

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“…In the setting of a right ventricle with little apparent ability to generate antegrade pulmonary blood flow and a restrictive ductus potentially limiting retrograde perfusion of the lungs, the team was sufficiently concerned regarding the potential inadequacy of pulmonary blood flow that our NICU "pulmonary hypertension" protocol was planned and initiated with the use of intubation, sedation, and low stimulation infant handling [22][23][24]. Inhaled nitric oxide therapy was started on arrival at the ICU [22,25], and though our pulmonary hypertension team was on standby, as was the ECMO team, adequate pulmonary blood flow proved relatively easy to achieve.…”

Section: Discussionmentioning

confidence: 99%

Management of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many—A Case Report

Kavanaugh-McHugh,

Zuckerwise,

Killen

et al. 2024

JCDD

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Collaborative multicenter research has significantly increased our understanding of fetal Ebstein anomaly, delineating risk factors for adverse outcomes as well as predictors of postnatal management. These data are incorporated into prenatal care and therapeutic strategies and inform family counseling and delivery planning to optimize care. This report details the translation of findings from multicenter studies into multidisciplinary prenatal care for a fetus with Ebstein anomaly, supraventricular tachycardia, and a circular shunt, including transplacental therapy to control arrhythmias and achieve ductal constriction, informed and coordinated delivery room management, and planned univentricular surgical palliation.

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Section: Discussionmentioning

confidence: 99%

Management of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many—A Case Report

Kavanaugh-McHugh,

Zuckerwise,

Killen

et al. 2024

JCDD

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“…Currently, the clinical treatment strategy for PPHN is focused on mechanical ventilation to maintain systemic circulatory pressure and reduce pulmonary hypertension (41). Neonatal care, including the use of inhaled NO and other vasodilators, improves the prognosis of infants with PPHN (42). However, clinical statistics show that ≤ 30% of newborns affected by PPHN do not respond to this treatment and require invasive life support measures (5).…”

Section: Discussionmentioning

confidence: 99%

LncRNA H19 improves angiogenesis in mouse pulmonary artery endothelial cells by regulating the HIF-1α/VEGF signaling pathway

Dou,

You,

Chai

et al. 2024

Preprint

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Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of acute respiratory failure characterized by systemic hypoxemia and elevated pulmonary arterial pressure, which leads to pathological changes in pulmonary vascular remodeling and endothelial cell function. Long non-coding RNA (lncRNA) H19 has been shown to be involved in the regulation of arterial endothelial cell function, but its regulatory role in PHN is not fully understood. In the present study, mouse pulmonary artery endothelial cells (MPAECs) were cultured in a hypoxic environment. Subsequently, the regulatory function of lncRNA H19 on MPAECs was explored by constructing adenoviruses knocking down and overexpressing lncRNA H19. The results revealed that the hypoxic environment could induce the proliferation and migration of MPAECs, as well as the high expression of lncRNA H19 in MPAECs. Knockdown of lncRNA H19 expression in MPAECs reversed hypoxic environment-induced functional changes in endothelial cells, whereas overexpression of lncRNA H19 further enhanced the proliferation and migration of MPAECs. In addition, further assays revealed that lncRNA H19 upregulated the hypoxia-inducible factor-1α (HIF-1α)/vascular endothelial growth factor (VEGF) pathway through sponge adsorption of microRNA-20a-5p, which in turn promoted changes in endothelial cell function. LncRNA H19 may interfere with vascular remodeling in hypoxia-induced pulmonary hypertension by upregulating the expression of HIF-1α and VEGF in vascular endothelial cells.

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“…PPHN is secondary to a number of underlying causes associated with impaired relaxation of the pulmonary vasculature, such as perinatal asphyxia, infection, and meconium aspiration syndrome (MAS) [ 6 – 8 ]. Elevated PVR leads to decreased pulmonary blood flow (PBF), resulting in hypoxemia and acidosis.…”

Section: Introductionmentioning

confidence: 99%

“…Postnatal increase in oxygen tension in the lungs is one of the most crucial factors to promote pulmonary vasodilatation [ 9 ]. Therefore, supplemental oxygen therapy is the mainstay in the treatment of PPHN to reduce PVR and increase oxygenation [ 6 – 8 ]. Inhaled nitric oxide (iNO) is a selective pulmonary vasodilator and is considered the first-line PPHN-specific therapy when oxygen therapy alone is insufficient to reduce PVR [ 6 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

Occurrence of hyperoxia during iNO treatment for persistent pulmonary hypertension of the newborn: a cohort study

de Jager,

Brouwer,

Reijman

et al. 2024

Eur J Pediatr

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High concentrations of oxygen are often needed to optimize oxygenation in infants with persistent pulmonary hypertension (PPHN), but this can also increase the risk of hyperoxemia. We determined the occurrence of hyperoxemia in infants treated for PPHN. Medical records of infants ≥ 34 + 0 weeks gestational age (GA) who received inhaled nitric oxide (iNO) were retrospectively reviewed for oxygenation parameters during iNO therapy. Oxygen was manually titrated to target arterial oxygen tension (PaO2) 10–13 kPa and peripheral oxygen saturation (SpO2) 92–98%. The main study outcomes were the incidence and duration of hyperoxemia and hypoxemia and the fraction of inspired oxygen (FiO2). A total of 181 infants were included. The median FiO2 was 0.43 (IQR 0.34–0.56) and the maximum FiO2 was 1.0 in 156/181 (86%) infants, resulting in at least one PaO2 > 13 kPa in 149/181 (82%) infants, of which 46/149 (31%) infants had minimal one PaO2 > 30 kPa. SpO2 was > 98% in 179/181 (99%) infants for 17.7% (8.2–35.6%) of the iNO time. PaO2 < 10 kPa occurred in 160/181 (88%) infants, of which 81/160 (51%) infants had minimal one PaO2 < 6.7 kPa. SpO2 was < 92% in 169/181 (93%) infants for 1.6% (0.5–4.3%) of the iNO time. Conclusion: While treatment of PPHN is focused on preventing and reversing hypoxemia, hyperoxemia occurs inadvertently in most patients. What is Known:• High concentrations of oxygen are often needed to prevent hypoxemia-induced deterioration of PPHN, but this can also increase the risk of hyperoxemia.• Infants with persistent pulmonary hypertension may be particularly vulnerable to the toxic effects of oxygen, and hyperoxemia could further induce pulmonary vasoconstriction, potentially worsening the condition. What is New:• Hyperoxemia occurs in the majority of infants with PPHN during treatment with iNO.• Infants with PPHN spent a considerably longer period with saturations above the target range compared to saturations below the target range.

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Evidence-Based Guidelines for Acute Stabilization and Management of Neonates with Persistent Pulmonary Hypertension of the Newborn

Cited by 8 publications

References 116 publications

Management of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many—A Case Report

Management of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many—A Case Report

LncRNA H19 improves angiogenesis in mouse pulmonary artery endothelial cells by regulating the HIF-1α/VEGF signaling pathway

Occurrence of hyperoxia during iNO treatment for persistent pulmonary hypertension of the newborn: a cohort study

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